Dubin-Johnson syndrome

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:

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Overview

Dubin-Johnson syndrome is an autosomal recessive disease which presents shortly after birth with an increase of conjugated bilirubin without elevation of liver enzymes (ALT, AST).

Dubin-Johnson syndrome
Bilirubin.png
Bilirubin
ICD-10 E80.6
ICD-9 277.4
OMIM 237500
DiseasesDB 3982
eMedicine med/588 
MeSH D007566

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Dubin-Johnson syndrome from Other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications, and Prognosis

Natural History

Complications

Prognosis

Prognosis is good and there's no need to treat this syndrome.

Diagnosis

Diagnostic Criteria

History and Symptoms

Physical Examination

Laboratory Findings

Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

Prevention

There is a lack of canalicular multi-drug resistant protein which causes dysfunction on bilirubin transfer to bile canaliculi.

An isoform of this protein is localized to the lateral hepatocyte membrane, allowing transport of glucuronide and glutathione conjugates back into the blood.

Analysis of urine pophyrins show a normal level of coproporphirin but the I isomer accounts for 80% ot the total(normally 25%)

Liver will present with dark brown appearance due to pigment accumulation.

See also

References

de:Dubin-Johnson-Syndrom




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